Motility Disorder Of Oesophagus

7/29/2019 Motility Disorder Of Oesophagus

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Motility disorder of

esophagus

• Dr Kapileshwer Vijay



ANATOMY & PHYSIOLOGY

Three physiologically distinct neuromuscular units

• Upper Esophageal Sphincter

• Esophageal Body

• Lower Esophageal Sphincter



Motility Disorders

• Upper esophageal

– UES disorders

– neuromuscular disorders

• Esophageal body

–

achalasia – diffuse esophageal spasm

– nutcracker esophagus

– nonspecific esophagealdysmotility

• LES

– achalasia

– hypertensive LES

• Primary disorders

– achalasia

– diffuse esophageal spasm

– nutcracker esophagus

– nonspecific esophagealdysmotility

• Secondary disorders

– severe esophagitis

– scleroderma

– diabetes

– Parkinson’s

– stroke



UES Disorders

• Cricopharyngeal hypertension

– elevated UES resting tone

– poorly understood (reflex due

to acid reflux or distension)

• Cricopharyngeal achalasia – incomplete UES relaxation

during swallow

– may be related to Zenker’s

diverticula in some patients

• Clinical manifestations

– localizes as upper (cervical)

dysphagia

– within seconds of swallowing

– coughing, choking, immediateregurgitation, or

nasal regurgitation

• Diagnosis: swallow evaluation &

modified barium swallow

• Treatment - Myotomy



ACHALASIA CARDIA

HISTRORICAL ASPECTS

• Greek word- failure to relax

• First described in 17 th century by Thomas

Willis : used whale bone for dilation.

• 1880 – Von Miculicz : cardiospasm as the

mechanism.

• 1957 – Code : manometric abnormalities in

Achalasia.



ACHALASIA CARDIA - PATHOGENESIS

• ETIOLOGY – unknown

• FAMILIAL

• AUTO-IMMUNITY: – Inflammatory infiltrate in myenteric

plexus , predominantly of T-Lymphocyte.

–High prevalence of class II HLAAntigens – DQ –W1, DQ-A1*0101

– Antibodies to myenteric plexus

neurons.

• Infective – Trypanosoma cruzi

–After an attack of Varicella-Zoster

– Association with Guiilain-Barresyndrome



Pathophysiology

• T‐lymphocyte, eosinophil, and mast cellinfiltration in the myenteric (Auerbach) plexus

Myenteric neural fibrosis• Hypertrophy of the two muscle layers and nerve

fibers

• Degeneration of NO and producing inhibitoryneurons

• Affects relaxation of LES Basal LES pressure rises



OVERVIEW OF ETIOPATHOGENESIS

Initial Insult

??viral

2° degeneration of vagalefferent neurones and their

cellbodies in DMN

Inflammatory Plexopathy primarilyinvolving NO Nerves

Vigorous AchalasiaDestruction of NO

Neurones

Classic Achalasia2° mucosal and submucosal

inflammation andesophageal dilatation

2° smooth musle

hypertrophy and

degeneration

?DES

?Nutcracker



- EPIDEMIOLOGY

• Incidence – 6:100 000

• Sex Ratio – Equal

• Age group – 5th to 6th decade

•Hereditary – AR

• Associated disease

– Chagas` DiseaseCardiomyopathy

– Cerebellar ataxia with MEN II

– TRIPLE “A” : Alacrimia,

Achalasia, Adrenal failure.

VENTRAPPEN - CLINICALCLASSIFICATION

1. None

2. Short-lasting episodes of

dysphagia and retrosternalpain < once a week

3. Dysphagia > once a week,

but no regurgitation or

weightloss

4. Frequent dysphagia

accompanied by weightloss

or regurgitation

ACHALASIA CARDIA



Typical

• Dyspahgia – To both solids andliquids, may be intermittent

- very slowworsening,indolentcourse

• Regurgitation – of bland,undigested food, hours aftermeal

Atypical

• Chest Pain(50%)

• Heartburn – Production of

lactic acid• Weight loss – may be

profound

• Recurrent aspirationpneumonia

ACHALASIA CARDIA – CLINICAL FEATURES



• PRIMARY EVALUATION

– Barium swallow withfluroscopy

– Esophageal Manometry

– Upper GI Endoscopy

Further evaluation forsecondary achalasia

–

Abdominal and thoracicCT

– Endoscopic Ultrasound

– Endoscopic brushings of GE Junction for cytology

ACHALASIA CARDIA - DIAGNOSIS



ACHALASIA CARDIA - BARIUM



ACHALASIA CARDIA

• FLUOROSCOPIC ABNORMALITIES

– Earliest : Breakdown of normal peristalsis into simultaneouscontractions

– Failure of primary peristaltic wave to clear barium

• VIDEOESOPHAGOGRAPHY

– Absence of normal peristalsis in esophageal body

– Incomplete or absent opening of LES / dilatation of esophagus

–100% correlation with manometry.



Manometery

• Gold standard

• Characteristic featuresregardless of the stage

– Aperistalsis is the

prerequisite.

– Sphincter pressure can benormal in up to 20-30%.

– Sphincter Relaxation maybe complete but of shortduration (< 6 sec) andfunctionally inadequate.



• To exclude other entities.• Normal mucosa, mild resistance

to passage of scope.

• Failure of relaxation of LES

with air insufflation

• Residual food / fluid

• Dilatation & tortuosity

• Careful retroflexed view TO EXCLUDE CARDIA GROWTH (2-4%)

– Marked resistance to passage of scope.(better appreciated with bigger scope)

ACHALASIA CARDIA - ENDOSCOPY



• EUS : High frequency 20 Hz

– Thickness of folds

– Submucosal tumor infiltration

– Regional lymphadenopathy

• CECT : - Asymmetric wall thickening

- Extrinsic mass / lymphadenopathy

ACHALASIA CARDIA – OTHER TESTS



Secondary / Pseudo-achalasia

• Benign : Amyloidosis

Peptic strictures

Post vagotomy effect

• Malignancy :

• Carcinoma stomach, esophagus, pancreas, HCC,Lung, Kidney, breast, prostate.

• Lymphoma

• Peritoneal Mesothelioma



Achalasia - Treatment

• AIM : Reducing the pressure gradient – relievingsymptoms and preventing further dilatation

• Only palliative

– Pharmacological

– Botulinum Toxin

– Pneumatic Balloon dilatation

– Myotomy

• Open

• Laparoscopic• Thorocoscopic



Pharmacological therapy• Nitrates : Isosorbide dinitrate

– c GMP

–

5-10mg S/L before each meal. – Onset – 15``, effect lasts for 90 ``.

– Relief in 75-80% But side effects in 30%

• Calcium Channel Blockers : Nifedipine, Diltiazem

– Reduce pressure by 40%, lasts > I hr.

– Effective in 70%

– RCT fail to show any benefit.

• COMPARATIVE TRIAL :

– Iso-sorbide > Nifedipine > Other CCB

85% 50%



Botulinum Toxin

• Inhibits the Ca++ dep. release of Ach. from nerve terminals.

• Effective in 85% but relapse in 50% in 6 months, due to regeneration of

nerve endings.

• Each vial 100 units

• 80-100 units, in a dilution of 20 units/mL (Lethal dose – 2000u)

• 1 cm above the ‘Z’ line in four quadrants.

• Candidates – older (>50 yrs)

- Vigorous acalasia

- High surgical risk

• Remarkable safety : mild chest discomfort

Reflux < 5%

• Gender/ age/ previous dilations DO NOT predict response rate



Botulinum Toxin First 6 months

First injection

Immediate Failure 10% Response 90%

30%Early Relapse

Further injections

10%

FAILED 30% IMPROVED 70%

Mean Remission : 1.4 yr(5 month. to 2.5 yrs)

20%



Pneumatic dilation

• The most effective non-surgical treatment

• Balloon across GE Jn. to tear the circular muscle fibres.• Rigiflex Balloon dilator(Microvasive)

– Double lumen catheter with low compliance polyetylenecylindrical balloon (through the scope)

–

3.0, 3.5, 4.0 cms• Witzel Dilator

– Polyurethane balloon mounted on a forward – viewendoscope. (Balloon inflation under direct vision withoutfluoroscopy)

– 3 psi 2-3 min.

• Efficacy 50-93 %



COMPLICATIONS OF PNEUMATIC DILATION

– Perforation - 3.3%

– Aspiration - 0.8% – Death - 0.2%

– Haemorrhage - < 0.1%

–

Reflux - 2% (0-9)• Requirement for each subsequent dilation decreases

effectiveness by 50%

• Surgery after 3 unsuccessful dilations

• Initial good response predicts response to subsequent

dilations

• Peristalsis may return in 20%



MYOTOMY

• Anterior myotomy down to the mucosa till the proximalextent of the LES and 1-2 cm on to the stomach.( 5mm)

• Combination with an anti-reflux procedure is debatable -

loose Nissen fundoplication

- Incomplete Toupet

- Dor Fundoplication

• 80-100% effective, Maintained at 10 years – 65%

•

14% eventually require esophagectomy.• Uncontrolled GERD in 10%(Higher with pH studies)



Surgery Or Balloon ?

• Efficacy studies

– RCT 100 pts 15 yr follow up

• Favours surgery ( Scendes, Surgery, 1998)

• Cost effectiveness analysis :

– 45 pts follow-up 7 years

• Favours dilation cumulative cost 2.5 times less.

( Parkman, Dig Dis Sci 1993)



ALGORITHM

PT. With Achalasia

Botulinum toxin 80-100units

pneumatic dilatation

Lap. Myotomy

High Surgical Risk Low surgical risk

FailureFailure Failure Success

Success

SuccessSuccess

Repeat Botulinum toxin

FailureFailure

Graded pneumatic dilatation

Nifedipine / NitratesEsophagectomy



Addition of Fundoplication

• Richards et al: Randomizedtrial

• Heller myotomy w/ Dor vsw/o Dor ( n = 43)

• Post‐op GERD (by 24‐hr pH

monitoring)• 47.5% in pts w/ Heller

myotomy alone

• 9.1% in pts w/ added Dorfundoplication

•

No difference in LES pressureor dysphagia scores

• Rice et al: Retrospective study

• Heller with and w/o Dor (n =149)

• Decreased incidence of GERD(by 24‐hr pH monitoring)

following fundoplication• Fundoplication did not

decrease esophageal emptyingtime

(assessed by barium

esophagography).

1. Richards WO, et al. Heller myotomy versus Heller myotomy with Dor fundoplication for achalasia: a prospective

randomized double-bline clinical trial. Ann Surg 2004; 240(3):405-415.

2. Rice TW, et al. A physiologic clinical study of achalasia: Should Dor fundoplication be added to Heller myotomy?. J

Thorac Cardiovasc Surg 2005; 130(6):1593-1600



DIFFUSE ESOPHAGEAL SPASM

• Osgood 1889 – severe chest pain and dysphagia.• Fleshler 1967- DES syndrome

• Familial Clusters

• ETIOPATHOGENESIS :

1. Defect in neural inhibition2. Decreased availability of nitric oxide.

3. Hyper sensiticity to cholinergic agents and pentagastrin stimulation

precipitating chest pain and dysphagia

4. Gastroesophageal reflux (20-50%)

5.Stressful life events.



CLINICAL FEATURES :

• Recurrent chest pain : - Variable in intensity, frequency and

location – Indistinguishable from cardiac chest pain.

– Most reliable

» Radiation through back

» Variability in amount of exercise precipitatingthe pain

» Associated dysphagia

• Dysphagia : intermittent, non – progressive

–To both liquids and solids, never severe to cause weight loss

– Ppt. By stress, rapid eating, liquids of extreme tempetature.

• Associated fetures of IBS




BARIUM STUDY

• Most commonly, Normal

• Disruption of peristalsis

• Tertiaty activity producing segmentation.• Results vary from day to day

• Spastic activity does not correlate with the symptoms.




Diffuse Esophageal Spasm

• Numerous nonpropulsive

contractions

• “corkscrew/ rosary bead”

esophagus

• DES requires normal peristalsisinterspersed with 30% +

periods of nonpropulsive

motor activity

• Segmental spasm

• Pseudodiverticulosis






MANOMETRY

• Simultaneous contractions > 20% of wet swallows.

• Pressure exceeding 30 mm Hg.

• Long duration contractions

• Intermittent Normal peristalsis

• Repetitive waves (> 2 peaks)

• Spontaneous contractions

• High amplitude

•Ambulatory manometery





TREATMENT:

• Reassurance

• Treat underlying GERD

– Smooth muscle relaxants

– Nitroglycerin 0.4 mg Sub ling.

– Isosorbide dinitrate10-30 mg four times daily

– Dicycloverine 10-20 mg four times daily

• Calcium Channel Blockers : can decrease high

amplitude contractions but inconsistent – Nifedipine 10-30 mg four times daily

– Diltiazem 60 mg four times daily




TREATMENT:Psychotropic Drugs : Reduce the discomfort without any effect

on the motility

– Trazodone 100-150 mg once daily

– Imipramine 50 mg once daily

• Botulinum Toxin patients with

• Pneumatic dilation abn. LES relaxation

•Oesophageal myotomy. Delayed emptying.




Nut cracker esophagus

• Dirst described -1979

• Dominant complaint – chestpain

•Most common motilitydisorder in pt. with noncardiac chest pain.(27-48%)

• Dysphagia relativelyuncommon.

• Association with GERD andstress.

• Radiology- Normal

•

Endoscopy- – Low threshfold for pain

– Reproduced by ballondistention



MANOMETRIC CRITERIA

• NUTCRACKER ESOPHAGUS

– Normal peristalsis

– Increased amplitude of

contractions > 180 mmHg

–Increased duration of contraction > 6 sec.



treatment

• Medical

– CCB

– Phosphodiesterase

inhibitor

• Surgical

– Long myotomy



Hypertensive LES

• First described by Code 1960

• Sec to Achlasia

• Clinical

– Dysphagia

– Chest pain

– GERD

– Somatization, nervous

• Radiology

– Ba

• Narrowing of LES

•

Manometery – Normal peristalsis

– LES Pressure > 45 mmHg

– Normal relaxation



• Treatment

– Medical

• CCB/Nitrate/Botox

•

Surgical – LES Myotomy with

partial fundoplication



• Contraction wave of < 30 mm Hg in amplitude,

• Do not effectively transport and clear the bolus

• Non Specific Esophageal Motility Disorders.

–INEFFECTIVE ESOPHAGEAL MOTILITY

– HYPOTENSIVE LES

• Associated GERD and ENT complaints

• Could be secondary to chronic acid damage to distalesophagus.

HYPO-CONTRACTING ESOPHAGUS



HYPOCONTRACTING ESOPHAGUS

Clinical feature

• Mild dysphagia

• Heartburn and acid

regurgitation dominant

symptoms.

• Severe dysphagia – peptic

stricture / esophagitis.

TREATMENT

• Proton Pump Inhibitors.

• Prokinetics.

• Results unreliable



Thanks

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Motility Disorder Of Oesophagus